Begley D, Mohiddin S, Fananapazir L
Inherited Heart Diseases Section, Cardiology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892, USA.
Pacing Clin Electrophysiol. 2001 Nov;24(11):1639-44. doi: 10.1046/j.1460-9592.2001.01639.x.
Intracavitary LV obstruction is an important determinant of clinical outcome in hypertrophic cardiomyopathy (HCM). In a minority of patients the obstruction is at the level of the papillary muscles. Mid-cavity obstructive HCM may be associated with a distal LV aneurysm and a worse prognosis. It is often not amenable to standard cardiac surgeryfor LV outflow obstruction. The long-term effects (mean follow-up 4.8+/-2.9 years) of dual chamber (DDD) pacemaker therapy in 14 patients with mid-cavity obstructive HCM (mean age 34+/-16 years, range 15-65 years) were studied. Patients were evaluated by cardiac catheterization at baseline and 6 months to 1 year after receiving DDD pacemakers off all drug therapy. Symptoms were improved in all patients and NYHA functional class reduced from 2.8+/-0.1 to 1.9+/-0.4 (P < 0.0005). Intracavitary LV pressure gradients was reduced significantly (43+/-36 vs 84+/-31 mmHg at baseline, P < 0.0005). There was a significant associated reduction in apical LV systolic pressure (152+/-37 vs 188+/-34 mmHg, P < 0.001). In addition, there was a trend towards increased exercise tolerance (445+/-123 vs 396+/-165). Cardiac output and LV filling pressures were unchanged. In conclusion, chronic DDD pacing results in significant symptomatic and hemodynamic improvement in this uncommon but important subset of patients with obstructive HCM in whom the role of cardiac surgery is less well defined compared with the more typical outflow tract location of LV obstruction.
心腔内左心室梗阻是肥厚型心肌病(HCM)临床预后的重要决定因素。少数患者的梗阻发生在乳头肌水平。心腔中部梗阻性HCM可能与左心室远端动脉瘤及更差的预后相关。它通常不适用于治疗左心室流出道梗阻的标准心脏手术。我们研究了双腔(DDD)起搏器治疗14例心腔中部梗阻性HCM患者(平均年龄34±16岁,范围15 - 65岁)的长期效果(平均随访4.8±2.9年)。在基线时以及接受DDD起搏器且停用所有药物治疗后6个月至1年,通过心导管检查对患者进行评估。所有患者症状均有改善,纽约心脏协会(NYHA)心功能分级从2.8±0.1降至1.9±0.4(P < 0.0005)。心腔内左心室压力梯度显著降低(基线时为43±36 mmHg,治疗后为84±31 mmHg,P < 0.0005)。左心室心尖部收缩压也显著降低(152±37 mmHg vs 188±34 mmHg,P < 0.001)。此外,运动耐量有增加的趋势(445±123 vs 396±165)。心输出量和左心室充盈压未改变。总之,对于这种罕见但重要的梗阻性HCM患者亚组,与更典型的左心室流出道梗阻部位相比,心脏手术的作用尚不明确,慢性DDD起搏可导致症状和血流动力学显著改善。